Matenda a Zellweger ndi matenda omwe sagwidwa ndi matenda, omwe amachititsa kuti peroxisomes, organelles, pafupifupi pafupifupi maselo onse a thupi, ayambe kugwira ntchito. Peroxisomes ndi omwe amachititsa maselo ambiri ofunika, kuphatikizapo mphamvu yamagetsi, zomwe zikutanthauza kuti matenda a Zellweger akhoza kukhudza thupi. Phunzirani za momwe matenda a Zellweger amakhudzira thupi, kuphatikizapo chithandizo ndi njira zothandizira uphungu.
Zellweger Spectrum Disorders
Matenda a Zellweger ndi matenda oopsa kwambiri omwe amatchedwa Zellweger spectrum disorders. Ngakhale kuti zovuta zapaderazi zimaganiziridwa kuti ndizosiyana, sizili m'gulu la mitundu yosiyanasiyana ya matenda. Zellweger spectrum matendawa ndi awa:
- Cerebrohepatorenal Syndrome
- Hyperpipecolic acidemia
- Matenda a Infantile Refsum
- Neonatal Adrenoleukodystrophy
- Zellweger Syndrome
Matendawa amakhala ndi zizindikiro zambiri, koma sikuti anthu onse adzakhala ndi zizindikiro zofanana kapena zotsatira zake malingana ndi kumene akugwera.
Zizindikiro
Matenda a Zellweger akuyembekezeka kuchitika pa 1 mwa onse 50,000 mpaka 100,000 obadwa. Amuna ndi akazi akhoza kubadwa ndi matendawa. Zimakhudza mbali zambiri za thupi, monga:
- Mutu ndi nkhope: mutu wotukulidwa; mphumi yakulu; lalikulu anterior fontanelle ("malo osafewa"); zopweteka zopweteka; nkhope yonyezimira
- Ubongo ndi ndondomeko zamanjenje: chitukuko chosasintha cha ubongo chomwe chimapangitsa kuvulala ; kumva ndi kusokonekera kwa masomphenya; kuchepetsa maganizo ndi kuchedwa kwachitukuko; kuchepa kapena kupezeka
- Chiwindi: chiwindi chofutukuka ndi zovuta; jaundice
- Impso: ziphuphu zamphongo; hydronephrosis
- Minofu ndi mafupa: kutsika kwambiri kwa minofu (hypotonia); zofooka za mafupa m'manja, miyendo, ndi mapazi
Kudziwa
Mutu ndi nkhope ya mwana wakhanda wobadwa ndi matenda a Zellweger zimapereka chitsimikizo kwa matendawa. Matenda a Zellweger amachititsa kuti mafuta aatali kwambiri awonjezeke (VLCFA), choncho mayesero a VLCFA angathandize kutsimikizira kuti akudwala matendawa. Izi ndi zina zamakono kwambiri za sayansi ndi majini amatha kuyesa malo ena oyesera.
Chithandizo
Ngakhale kuti kafukufuku wapita patsogolo pomvetsa matenda a Zellweger, palibe chithandizo chomwe chilipobe, ndipo makanda obadwa ndi matendawa amafa m'chaka choyamba cha moyo. Chithandizo chamankhwala chimayang'ana kwambiri pochiza zizindikiro zomwe zilipo, monga chiwindi chosagwira ntchito ndi kugwidwa. Kusintha kuchuluka kwa VLCFA mu zakudya sikunasonyezedwe kukhala mankhwala othandiza.
Kuphatikiza apo, mankhwala, thupi, ntchito, ndi kulankhula zingathandize podyetsa ndi kutonthoza.
Uphungu Wachibadwa
Kuzindikira koyamba kwa matenda a Zellweger ndi mavuto ena a Zellweger spectrum ndizotheka kupyolera mwa kuyesedwa kwa majini. Matenda a Zellweger amaloledwa mwachidziwitso, kutanthauza kuti ana amakula ngati makolo onsewo akunyamula jini lolakwika. Ngati ndi choncho, mwana aliyense wamtsogolo ali ndi mwayi wokwanira wobadwa ndi matenda a Zellweger.
Aphungu amtunduwu akhoza kukuthandizani kuti mukulankhule ndi chiopsezo chanu.
Zotsatira:
Chedrawi, A, & Clark, G. (2002). Matenda a peroxisomal. eMedicine.
Grayer, J. (2005). Kuzindikira kwa matenda a Zellweger ali wakhanda. Adv Neonatal Care, 5 (1), 5-13.